Ultrasound biomicroscopy-based diagnosis and cryotherapy for idiopathic rhegmatogenous retinal detachment secondary to pars plicata non-pigmented epithelium break: a case description

Introduction

Rhegmatogenous retinal detachment (RRD) generally refers to retinal detachment (RD) caused by a neurosensory retina break or hole. The first reported case of ciliary body non-pigmented epithelium (NPE) detachment combined with RD was in 1953, which was a postoperative patient with a scleral rupture and partial dislocation of the lens (1). Similar cases were subsequently reported related to atopic dermatitis (AD) or Schwartz-Matsuo syndrome.

Breaks in the ciliary body combined with RD in patients with AD were identified in the reports, demonstrating that breaks were located mainly in the pars plana, followed by the pars plicata. Developmental abnormalities of lens and zonules were often associated with these cases (2,3). Schwartz-Matsuo syndrome was first reported in 1973 and was characterized by RRD, aqueous cells and flare, and high intraocular pressure (IOP). The most common cause is trauma, and RRD in this syndrome usually results from retinal dialysis and breaks in the pars plana and pars plicata (4).

RRD can be diagnosed by many examinations, such as B-scan ultrasonography and optical coherence tomography (OCT) (5). OCT can offer high-resolution retinal images, help determine whether the macula is involved, and demonstrate retinal interlayer structure. However, it is relatively expensive and has a limited field of view (6). Ultrasound biomicroscopy (UBM) is advanced in revealing peripheral breaks, especially those in the ciliary body. It can also diagnose ciliochoroidal detachment, which can help determine the severity of the RRD. These advantages are not easily matched by OCT due to the light attenuation by the sclera and pigmented layers (7,8).

This case report describes a patient from China with unilateral RRD characterized by a pars plicata NPE break. There was no history of trauma or evidence of AD in this case. Following transconjunctival cryotherapy and pneumatic retinopexy under the guidance of UBM, the retina was reattached successfully.

Case presentation

A 24-year-old Chinese female presented with a chief complaint of progressive decreased vision in her left eye for three weeks. Nasal visual field defect, distorted vision, and intermittent eye pain were also present. She had previously been diagnosed with RD, but no retinal breaks were found during a previous examination at another hospital. She had been asked to refer to our hospital for further diagnosis and treatment. The patient and her family denied any history of trauma, systemic or eye diseases, such as AD and glaucoma.

The best corrected visual acuity (BCVA) at the patient’s first visit was 20/20 in the right eye and 20/100 in the left. The IOP in both eyes was 22 mmHg, slightly higher than normal. Slit-lamp examination showed smooth and clear corneas of both eyes, and no abnormalities were observed in the anterior chamber. The lens of the right eye was clear, but there was mild posterior capsular opacification in the left eye. The B-scan ultrasonography confirmed RD, and dilated fundus examination revealed RD from the 1 to 4 o’clock direction in the superior-temporal quadrant without obvious causative breaks within the detachment area (Figure 1). Ultra-widefield OCT (UWF-OCT) revealed detachment of retinal nerve fiber layer (RNFL) from retinal pigment epithelium (RPE), which involved part of the macula (Figure 1B). Both UWF-OCT and detailed fundus examination with a three-mirror lens could not find any breaks. Fortunately, the UBM demonstrated a small NPE break at the pars plicata, at about 2 o’clock in direction (Figure 1D). We attempted to use anterior segment OCT (AS-OCT) for further examination, but failed to locate the break.

Figure 1 The preoperative ophthalmic examinations of the patient. (A) B-scan ultrasonography; (B) optical coherence tomography revealed macula-involving retinal detachment (blue star) and retinal microcysts (blue arrows); (C) scanning laser ophthalmoscopy revealed retinal detachment from the 1 to 4 o’clock (white arrows); (D) ultrasound biomicroscopy revealed a break at the pars plicata (white circle).

To diminish surgical damage, we conducted many preoperative discussions and ultimately decided to prioritize transconjunctival cryotherapy combined with pneumatic retinopexy under local anesthesia. We re-examined the UBM preoperatively and marked the most apparent direction of the break to assist our surgery. During the surgery, anterior chamber paracentesis and aqueous humor drainage were first performed. Since the break could not be observed directly, even under scleral indentation, we conducted localized transconjunctival cryotherapy under the guidance of UBM, which was at about 2 o’clock, 2 mm from the limbus, and lasting about 30 seconds. In the end, intraocular long-acting inert gas was injected (GOT Multi C3F8; ALCHIMIA Srl, Viale Austria, Italy). Unexpectedly, on the first day after surgery, the patient exhibited a psychiatric condition and refused to cooperate with the postoperative examination. It was not until this moment that the patient’s family informed us that the patient had a history of symptoms such as mutism and depression, but not of any self-harming behavior such as self-slapping or eye-striking. Furthermore, these symptoms had not been diagnosed or treated. Therefore, after ensuring that the patient did not require further urgent treatment, we agreed to the family’s request for discharge from the ophthalmology department. As a result, we could not obtain short-term postoperative information such as IOP and fundus manifestations.

Six days later, after the mental symptoms had improved, the patient underwent a postoperative follow-up. Fundus examination showed that the subretinal fluid was completely absorbed, and the inert gas occupied approximately one-quarter of the vitreous cavity. The OCT showed that the macula had reattached, but defects in the photoreceptor segments remained. It also revealed a tendency of posterior vitreous detachment (PVD). The UBM could not clearly visualize the condition of the break due to artifacts caused by the inert gas. Two weeks postoperatively, the BCVA of the left eye recovered to 20/215, and the IOP was 22 mmHg. The UBM showed that the NPE at the pars plicata had reattached, and the break was closed (Figure 2A). The OCT confirmed the PVD. Fundus examination revealed the residual inert gas occupying approximately one-fifth of the vitreous cavity and two new retinal breaks in the inferior retina. Therefore, retinal laser photocoagulation was performed to seal the breaks at 4 and 5:30 o’clock (Figure 2). After a further two weeks, the BCVA further recovered to 20/80 in the left eye. However, keratic precipitates (KPs) and aqueous flare reoccurred. The iris appeared slightly gathered at the upper temporal quadrant, where cryotherapy was performed, and a small amount of pigment was deposited on the posterior capsule of the lens. Fundus examination showed a small amount of residual inert gas, and two breaks in the inferior retina were well-closed with clearly defined laser spots around. The UBM revealed a stable condition in the area of pars plicata that had been treated. The IOP of the left eye elevated to 25 mmHg, and we intensified the anti-inflammatory treatment with Pred Forte (Allergan, Inc., Irvine, CA, USA) and Bronuck (Senju Pharmaceutical Co., Ltd. Osaka, Japan). Seven weeks postoperatively, the BCVA was maintained at 20/80 in the left eye due to the macular involvement. The anterior chamber inflammation (ACI) disappeared, and IOP decreased to the normal range. The condition was stable at this time, and the patient felt good about herself. She was advised to follow-up if there were any changes.

Figure 2 The postoperative ophthalmic examinations of the patient. (A) Ultrasound biomicroscopy; (B) scanning laser ophthalmoscopy and two breaks sealed by laser (white circles); (C) optical coherence tomography.

All procedures performed in this study were in accordance with the ethical standards of the institutional and/or national research committee(s) and with the Helsinki Declaration and its subsequent amendments. Written informed consent was provided by the patient for publication of this article and accompanying images. A copy of the written consent is available for review by the editorial office of this journal.

Discussion

RRD caused by pars plicata NPE break has been rarely reported. Some of the reported cases have had a history of AD and abnormalities of the lens and zonules (3,9). It has been speculated that the break may be due to slapping and rubbing the eyes, ectodermal dysplasia, inflammatory responses, and other factors (10,11). Reports have also indicated that pars plicata NPE break could be formed by the traction through the zonules after secondary cataract surgery (12). Similar conditions in systemic diseases, such as Stickler syndrome, have also been reported (13). Others have been categorized into Schwartz-Matsuo syndrome, which is characterized by RRD, aqueous cells and flare, and high IOP. The common causes are eye trauma and surgery (14). The aqueous flare and high IOP are confirmed to be due to the photoreceptor outer segments leaking into the anterior chamber through the breaks and obstructing the trabecular meshwork (15).

In this case, the IOP was slightly higher at the first visit, but there was no aqueous flare. Therefore, it could not be diagnosed as Schwartz-Matsuo syndrome. To our knowledge, there had been only one similar case in China, and that patient did not have a history of AD either. However, he was discovered to have developmental abnormalities of the lens-zonule-ciliary body complex postoperatively (16). In our report, the patient denied any history of AD, trauma, or self-harming behavior and was without any developmental abnormality or high IOP, making it an idiopathic RRD with pars plicata NPE break. It is noteworthy that our patient exhibited postoperative psychiatric abnormalities, such as mutism and depression, prompting us to consider the possibility of self-harm behaviors such as self-slapping or eye-striking. These behaviors, particularly involving the periorbital region, are potential causes of pars plicata NPE break. However, upon further inquiry, the patient’s family denied any history of self-harming behaviors. Throughout the follow-up period, we repeatedly recommended that the patient consult the relevant departments for further diagnosis and treatment, but the patient consistently refused. Therefore, we were unable to ascertain whether the patient had an underlying psychiatric issue or not. Moreover, considering such cases typically involve other traumatic ocular signs, such as lens dislocation, we still diagnosed this case as idiopathic rather than traumatic RRD.

UWA-OCT and UBM examinations are suggested to find the breaks that might be located in the extreme periphery of the retina or even the ciliary body. In our case, we failed to locate the break with routine or ultra-wide-field fundus examination, such as a scanning laser ophthalmoscope. The break could not be observed directly during the surgery, even under scleral indentation. So, in order to ensure the success of the operation, we had to locate the break with the auxiliary examination. AS-OCT and UBM were performed, and fortunately, UBM succeeded in revealing the break. The results were consistent with those reported in the relevant cases (17,18).

Most reports have suggested scleral buckling alone or in combination with cryotherapy, laser photocoagulation, or diathermy to seal the breaks in the ciliary body. However, these may lead to postoperative astigmatism, extrusion, and intrusion of implants (2,9,16,19). For patients with large breaks or combined with retinal traction, encircling buckling could be performed to reconstruct the artificial ora serrata (18). Vitrectomy combined with cataract surgery has been suggested for patients with lens abnormalities or complex conditions (3,20). In order to diminish the surgical damage, we performed a combination of transconjunctival cryotherapy and pneumatic retinopexy in this case. We interpreted that the anterior chamber paracentesis also played a role in the drainage of subretinal fluid besides adjusting the IOP, as the break could be considered connected to the anterior chamber due to its anterior location.

The surgery proved successful, with the break closed and retinal reattachment occurring within the first week postoperatively. Two new retinal breaks were found during the second week follow-up. We suspect that they might have been caused by the inert gas, which increased vitreous traction of the retina, and the OCT confirmed the PVD. At the 1-month follow-up, the IOP of the left eye was slightly elevated. We suspect this may have been due to ACI or a condition similar to Schwartz-Matsuo syndrome, for the slit-lamp examination found KP and aqueous flare. We suggested that the patient continue follow-up. The AS condition, whether there are new breaks and contralateral eye fundus, should be focused on in the follow-up.

Conclusions

This report was the first case in China to present an idiopathic RRD combined with an NPE break in the pars plicata. Moreover, it showed that transconjunctival cryotherapy combined with pneumatic retinopexy under the guidance of UBM could successfully treat ciliary body break. We recommend that patients who are strongly suspected of RRD but no breaks are found should undergo UWF-OCT and UBM examinations, regardless of whether the patient has a history of AD or trauma. For patients with a confirmed break, cryotherapy alone may be considered without scleral buckling to minimize surgical damage.

Acknowledgments

None.

Footnote

Funding: None.

Conflicts of Interest: All authors have completed the ICMJE uniform disclosure form (available at https://qims.amegroups.com/article/view/10.21037/qims-2025-329/coif). The authors have no conflicts of interest to declare.

Ethical Statement: The authors are accountable for all aspects of the work in ensuring that questions related to the accuracy or integrity of any part of the work are appropriately investigated and resolved. All procedures performed in this study were in accordance with the ethical standards of the institutional and/or national research committee(s) and with the Helsinki Declaration and its subsequent amendments. Written informed consent was provided by the patient for publication of this article and accompanying images. A copy of the written consent is available for review by the editorial office of this journal.

Open Access Statement: This is an Open Access article distributed in accordance with the Creative Commons Attribution-NonCommercial-NoDerivs 4.0 International License (CC BY-NC-ND 4.0), which permits the non-commercial replication and distribution of the article with the strict proviso that no changes or edits are made and the original work is properly cited (including links to both the formal publication through the relevant DOI and the license). See: https://creativecommons.org/licenses/by-nc-nd/4.0/.

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